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The multiple endocrine neoplasia (MEN) syndromes comprise 3 genetically distinct familial diseases involving adenomatous hyperplasia and malignant tumors in several endocrine glands. Clinical features depend on the glandular elements involved.
Each syndrome is inherited as an autosomal dominant trait with a high degree of penetrance, variable expressivity, and production of seemingly unrelated effects by a single mutant gene. The specific mutation is not always known.
Symptoms and signs develop at any age. Proper management includes early identification of affected individuals within a kindred and surgical removal of the tumors when possible. Although these syndromes are generally considered clinically distinct, significant overlap exists (see Table 1: Multiple Endocrine Neoplasia (MEN) Syndromes: Conditions Associated With MEN Syndromes ).
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Table 1
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Conditions Associated With
MEN Syndromes
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Condition
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MEN 1
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MEN 2A
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MEN 2B
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Parathyroid adenomas
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≥ 90%
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10–20%
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—
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Enteropancreatic tumors
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60–70%
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—
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—
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Pituitary adenomas
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15–42%
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—
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—
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Medullary thyroid carcinoma
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—
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> 90%
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> 90%
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Pheochromocytomas
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—
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50%
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60%
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Mucosal neuromas
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—
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—
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approx. = 100%
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Marfanoid habitus
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—
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—
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approx. = 100%
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MEN = Multiple endocrine neoplasia.
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Last full review/revision June 2008 by Patricia A. Daly, MD; Lewis Landsberg, MD
Content last modified June 2008
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